16例伴生长激素缺乏的多种垂体激素缺乏患者的临床特点及生长激素治疗效果分析

The characteristics and effects of growth hormone treatment of 16 multiple pituitary hormone deficiency patients with growth hormone deficiency

目的探讨伴生长激素缺乏(GHD)的多种垂体激素缺乏症(MPHD)患者的临床特点以及生长激素(GH)治疗效果。方法回顾性分析16例伴有生长激素缺乏的多种垂体激素缺乏患者的临床资料。结果本研究纳入了16例MPHD的患者,其中伴甲状腺功能减退症9例、伴低促性腺激素性性腺功能低减13例和伴肾上腺皮质功能减退6例。臀位、足先露和难产等不良生产史患者10例。骨龄(11.0±3.5)岁,明显落后实际年龄。L-Dopa-GH激发试验GH峰值为(0.14±0.17)ng/m L,GH治疗平均剂量(0.11±0.02)IU/kg。治疗后IGF-1水平及生长速度均明显增加。结论排除下丘脑、垂体占位等病变后,伴GH缺乏的MPHD患者在纠正其他轴系激素缺乏后,使用GH治疗可明显改善身高,并且无严重不良事件发生。

Objective To study the clinical feature and therapeutic effects of 16 patients diagnosed with multiple pituitary hormone deficiency with growth hormone deficiency （GHD）. Methods Clinical data of 16 cases were ret- rospectively studied. Results 16 MPHD with GHD patients were included, containing 9 hypothyroidism, 13 hypog- onadism and 6 ACTH deficiency. Ten of them had breech or foot presentation. The mean bone age was （ 11.0±3.5 ）years, delayed from chronological age, peak GH of L-Dopa provocation test was （0. 14 ±0. 17）ng/mL. GH dose was （0. 11 ± 0.02） IU/kg. After GH replacement therapy, the serum concentration of IGF- 1 and growth veloc- ity increased. Conclusions GH replacement therapy is crucial once after one is diagnosed of MPHD excluding the hypothalamus and pituitary lesions. Other pituitary hormones must be replaced sufficiently for MPHD patients. There is no severe adverse event.